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(American Journal of Pathology. 2001;158:2089-2096.)
© 2001 American Society for Investigative Pathology

Regular Article

A Distinctive Pediatric Renal Neoplasm Characterized by Epithelioid Morphology, Basement Membrane Production, Focal HMB45 Immunoreactivity, and t(6;11)(p21.1;q12) Chromosome Translocation

Pedram Argani^*, Anita Hawkins^*, Constance A. Griffin^*, Jeffrey D. Goldstein, Mark Haas^*, J. Bruce Beckwith, Carl B. Mankinen and Elizabeth J. Perlman^*

From the Department of Pathology,^*
The Johns Hopkins Hospital, Baltimore, Maryland; the Wolfson Children’s Hospital,
Jacksonville, Florida; Loma Linda University,
Loma Linda, California; and the Cytogenetics Laboratory,
the Nemours Children’s Clinic, Jacksonville, Florida

We report two cases of a hitherto undescribed pediatric renal neoplasm that is distinctive at the morphological, immunohistochemical, ultrastructural, and cytogenetic levels. On light microscopy, the tumors are composed of nests of polygonal, clear to eosinophilic cells associated with a subpopulation of smaller cells that surround hyaline material. Despite their epithelioid morphology, these tumors do not label immunohistochemically for epithelial markers but instead label focally for melanocytic markers HMB45 and Melan A. The hyaline material is positive with periodic acid-Schiff and methenamine-silver histochemical stains, and labels immunohistochemically for type 4 collagen. Ultrastructural examination confirms that it represents basement membrane material. Cytogenetic analysis reveals the identical t(6;11)(p21.1;q12) chromosome translocation as the sole abnormality in these two tumors, confirming their identity and distinctive nature.

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